|
|
Trans Am Ophthalmol Soc. 1980; 78: 226–242. | PMCID: PMC1312141 |
Treatment of gyrate atrophy of the choroid and retina with low arginine diet. R R McInnes, S A Arshinoff, L Bell, and C McCulloch Abstract In gyrate atrophy the blood ornithine is grossly elevated, due to deficiency of ornithine ketoacid transaminase, which converts ornithine towards glutamic acid. Two patients with gyrate atrophy have been treated with a low arginine diet and their blood ornithine levels have been reduced to near normal. At this level hyperammonemia may result from overtreatment, but this can be quickly cleared by a small dose of arginine. There has also been some improvement in vision, but no clearing of the gyrate areas. Future care with this regimen seems possible and improvements in handling of these patients are likely. Full text Full text is available as a scanned copy of the original print version. Get a printable copy (PDF file) of the complete article (2.8M), or click on a page image below to browse page by page. Links to PubMed are also available for Selected References. Images in this article Click on the image to see a larger version. These references are in PubMed. This may not be the complete list of references from this article. - Simell O, Takki K. Raised plasma-ornithine and gyrate atrophy of the choroid and retina. Lancet. 1973 May 12;1(7811):1031–1033. [PubMed]
- Takki K, Simell O. Genetic aspects in gyrate atrophy of the choroid and retina with hyperornithinaemia. Br J Ophthalmol. 1974 Nov;58(11):907–916. [PubMed]
- McCulloch JC, Arshinoff SA, Marliss EB, Parker JA. Hyperornithinemia and gyrate atrophy of the choroid and retina. Ophthalmology. 1978 Sep;85(9):918–928. [PubMed]
- Arshinoff SA, McCulloch JC, Matuk Y, Phillips MJ, Gordon BA, Marliss EB. Amino-acid metabolism and liver ultrastructure in hyperornithinemia with gyrate atrophy of the choroid and retina. Metabolism. 1979 Oct;28(10):979–988. [PubMed]
- Valle D, Kaiser-Kupfer MI, Del Valle LA. Gyrate atrophy of the choroid and retina: deficiency of ornithine aminotransferase in transformed lymphocytes. Proc Natl Acad Sci U S A. 1977 Nov;74(11):5159–5161. [PubMed]
- Trijbels JM, Sengers RC, Bakkeren JA, De Kort AF, Deutman AF. L-Ornithine-ketoacid-transaminase deficiency in cultured fibroblasts of a patient with hyperornithinaemia and gyrate atrophy of the choroid and retina. Clin Chim Acta. 1977 Sep 1;79(2):371–377. [PubMed]
- O'Donnell JJ, Sandman RP, Martin SR. Deficient L-ornithine: 2-oxoacid aminotransferase activity in cultured fibroblasts from a patient with gyrate atrophy of the retina. Biochem Biophys Res Commun. 1977 Nov 21;79(2):396–399. [PubMed]
- Shih VE, Berson EL, Mandell R, Schmidt SY. Ornithine ketoacid transaminase deficiency in gyrate atrophy of the choroid and retina. Am J Hum Genet. 1978 Mar;30(2):174–179. [PubMed]
- Weleber RG, Kennaway NG, Buist NR. Vitamin B6 in management of gyrate atrophy of choroid and retina. Lancet. 1978 Dec 2;2(8101):1213. [PubMed]
- Valle D, Walser M, Brusilow SW, Kaiser-Kupfer M. Gyrate atrophy of the choroid and retina: amino acid metabolism and correction of hyperornithinemia with an arginine-deficient diet. J Clin Invest. 1980 Feb;65(2):371–378. [PubMed]
- McCulloch C, Marliss EB. Gyrate atrophy of the choroid and retina: clinical, ophthalmologic, and biochemical considerations. Trans Am Ophthalmol Soc. 1975;73:153–171. [PubMed]
- Shih VE, Schulman JD. Ornithine-ketoacid transaminase activity in human skin and amniotic fluid cell culture. Clin Chim Acta. 1970 Jan;27(1):73–75. [PubMed]
- Phang JM, Downing SJ, Valle D. A radioisotopic assay for ornithine-delta-transaminase. Anal Biochem. 1973 Sep;55(1):272–277. [PubMed]
- Walser M, Batshaw M, Sherwood G, Robinson B, Brusilow S. Nitrogen metabolism in neonatal citrullinaemia. Clin Sci Mol Med. 1977 Aug;53(2):173–181. [PubMed]
- SNYDERMAN SE, BOYER A, HOLT LE., Jr The arginine requirement of the infant. AMA J Dis Child. 1959 Feb;97(2):192–195. [PubMed]
- ROSE WC, HAINES WJ, WARNER DT. The amino acid requirements of man. V. The rôle of lysine, arginine, and tryptophan. J Biol Chem. 1954 Jan;206(1):421–430. [PubMed]
- Simell O, Takki K. Raised plasma-ornithine and gyrate atrophy of the choroid and retina. Lancet. 1973 May 12;1(7811):1031–1033. [PubMed]
- Berson EL, Schmidt SY, Rabin AR. Plasma amino-acids in hereditary retinal disease. Ornithine, lysine, and taurine. Br J Ophthalmol. 1976 Feb;60(2):142–147. [PubMed]
|
